Antiphospholipid syndrome

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Antiphospholipid syndrome
Micrograph showing thrombotic microangiopathy, as may be seen in antiphospholipid syndrome
Synonyms Hughes syndrome
Pronounce N/A
Specialty N/A
Symptoms Thrombosis, recurrent miscarriage, stroke, deep vein thrombosis
Complications Pulmonary embolism, renal failure, heart attack
Onset Usually in adulthood
Duration Chronic
Types N/A
Causes Autoimmune disease
Risks Systemic lupus erythematosus, infections, medications
Diagnosis Blood test for antiphospholipid antibodies
Differential diagnosis Systemic lupus erythematosus, thrombophilia, disseminated intravascular coagulation
Prevention Anticoagulation in high-risk individuals
Treatment Anticoagulants such as warfarin, heparin
Medication Warfarin, heparin, aspirin
Prognosis Variable, depends on severity and management
Frequency 1-5 per 100,000 people per year
Deaths Can be fatal if not treated


Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of autoantibodies against phospholipid-binding proteins, most notably β₂-glycoprotein I and cardiolipin. These antibodies increase the risk of blood clot formation in both arteries and veins, as well as pregnancy complications such as recurrent miscarriage, stillbirth, and preeclampsia. APS may occur as a primary condition or in association with other autoimmune diseases, particularly systemic lupus erythematosus (SLE).

Pathophysiology[edit | edit source]

APS results from an immune-mediated attack on phospholipid-protein complexes. This leads to endothelial cell activation, platelet aggregation, and impaired fibrinolysis, which together promote a hypercoagulable state. The most commonly implicated autoantibodies are:

Signs and Symptoms[edit | edit source]

Symptoms of APS stem from thrombotic events and may include:

Venous blood clots in legs due to Antiphospholipid syndrome.

Diagnosis[edit | edit source]

Diagnosis is based on both clinical and laboratory criteria, evaluated on two occasions at least 12 weeks apart:

Diagnostic techniques include:

Types[edit | edit source]

  • Primary APS: Occurs in the absence of other autoimmune conditions.
  • Secondary APS: Occurs with conditions like SLE.
  • Catastrophic antiphospholipid syndrome (CAPS): A rare, life-threatening variant involving widespread microvascular thrombosis.

Neurological Features[edit | edit source]

Obstetric Manifestations[edit | edit source]

Treatment[edit | edit source]

APS is managed by preventing thrombosis and managing associated autoimmune disease:

Prognosis[edit | edit source]

With proper treatment, long-term outlook is generally favorable. Patients require lifelong monitoring, especially during pregnancy and surgical procedures. The risk of recurrence is significant if therapy is discontinued.

History[edit | edit source]

APS was first described by Dr. Graham Hughes in the early 1980s, hence its alternative name, Hughes syndrome.

See also[edit | edit source]

External links[edit | edit source]

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Contributors: Prab R. Tumpati, MD